**Dupuytren's Disease Dupuytren's Disease**

Jeremiah D. Johnson, Colin Pavano and Craig Rodner Jeremiah D. Johnson, Colin Pavano and Craig Rodner Additional information is available at the end of the chapter

Additional information is available at the end of the chapter

http://dx.doi.org/10.5772/intechopen.72759

#### **Abstract**

Dupuytren's disease is a fibroproliferative disease affecting the palmar fascia of the hand and leading to flexion contractures of the digits. It was first described in Northern European populations and derived its namesake from Dr. Baron Dupuytren, a French surgeon, who was one of the first to lecture on the disease. The etiology of Dupuytren's disease is unclear but is likely influenced by both genetic and environmental factors. Older individuals and men are most at risk of developing the disease. Dupuytren's disease is a clinical diagnosis and patients often present with gradually worsening flexion contractures. Mild disease is usually observed, but surgical treatment is preferred for debilitating contractures. A variety of surgical techniques have been described involving either incising or excising diseased fascia. Overall, surgery is effective in correcting contractures and improving function, but despite successful treatment some patients still experience recurrence. More recently, collagenase injections and percutaneous procedures have been utilized to treat Dupuytren's disease and have yielded promising results in select patients.

DOI: 10.5772/intechopen.72759

**Keywords:** Dupuytren's disease, collagenase, flexion contractures, palmar fascia, limited fasciectomy, percutaneous needle fasciotomy, fibroproliferative disease

#### **1. Introduction**

Dupuytren's disease is one of the most common pathologies diagnosed in the field of hand surgery. It is a fibroproliferative disease of the palmar fascia characterized by flexion contractures of the digits and involves an abnormal production of type III collagen. The disease has a unique history with origins dating back to the Vikings and has been studied extensively since. However, despite ongoing research the etiology of the disease remains unclear but likely results from a complex interaction between genetics and environmental risk factors. Patients classically present with palpable palmar nodules and cords leading to gradual, progressive loss of function. The disease can potentially lead to debilitating flexion contractures of the digits

© 2016 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. © 2018 The Author(s). Licensee IntechOpen. This chapter is distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

and affect activities of daily living. The disease is a clinical diagnosis and only requires further testing to exclude other pathologies. There is no cure for Dupuytren's disease and treatment methods remain palliative. Patients with mild disease can be observed for disease progression while patients with more severe disease may be treated with a variety of procedures or surgeries. Recently, clinical procedures including collagenase injections and percutaneous needle fasciotomy (PNF) have been utilized to successfully treat select patients. Still, surgery remains the preferred method of treating Dupuytren's contractures for most surgeons. A variety of surgical techniques have been described utilizing different types of incisions to either incise or excise disease fascia and correct contractures. Surgery has yielded successful outcomes in regaining extension of the involved digits and improving function of the hand, however, it is not without risks. Complications related to infection, wound healing, and neurovascular injuries have been reported. In addition, despite successful treatment following surgery some patients experience recurrence of their contracture. Further research has focused on methods of successfully treating Dupuytren's disease while reducing complications and recurrence. This chapter will provide a thorough description of Dupuytren disease from its history and pathophysiology to clinical management as well as highlight research related to patient outcomes.

as 1614 [2]. Despite possible earlier accounts, Dupuytren maintains the namesake of the disease to this day. Dupuytren finished his career as chief surgeon at the Hôtel de Dieu in Paris, and his name appears in at least 12 other diseases and instruments, cementing his legacy in

Dupuytren's Disease

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http://dx.doi.org/10.5772/intechopen.72759

Dupuytren's disease is often considered a "disease of the north," in to its northern European origins. Various studies into the prevalence of Dupuytren's disease demonstrate its northern roots, showing Norway, Scotland, and Iceland with some of the highest prevalence when compared to more southern nations [8–10]. Today, Dupuytren's disease can be seen in all types of patients; however, there are specific subsets of the population at in increased risk of

Dupuytren's disease is most prominent in Northern European white males, especially greater than 40 years old. A study in the Netherlands reports a prevalence as high as 22% in the general population [11]. The study also demonstrated a propensity for older populations with ages 50–55 displaying a 4.9% prevalence, while those 76–80 years old having a prevalence of 52.6%. Men were also affected disproportionately more than women (26.4 vs. 18.6%). The prevalence in the US has been shown to approach 7.3% when including self-reported symptoms [12]. Other epidemiological studies show a male to female ratio in the US of 1.7:1 which approaches 1:1 with increasing age [13]. Dupuytren's disease has been linked to both genetic and environmental factors, both of which contribute to the prevalence in patient populations throughout the world.

The genetic component of Dupuytren's disease has been a topic of interest for many years. A study by Burge et al. found that the prevalence of Dupuytren's in Norwegian individuals over 60 years old reaches 30%, indicating a familial component in like populations [14]. They also suggested an autosomal dominant inheritance pattern with variable penetrance based on pedigree analysis. Multiple heritable patterns have been hypothesized, but there is no clear consensus on a mode of transmission. It is possible the disease does not carry a simple inheritance pattern, but rather follows a more complex method similar to heart disease and diabetes. Ling et al. performed a study examining the family members of patients with Dupuytren's disease, and found that 53% of men and 33% of women over the age of 60 in the family had signs of the disease [15]. In a clinical study, patients with a family history of Dupuytren's disease had a 6-year earlier onset of disease compared to patients without a family history [16]. There was also increased disease severity in terms of the number of affected digits and degree of contracture in patients with a family history. Both of these findings suggest patients with a family history of Dupuytren's disease develop a more severe and earlier onset of disease. Research has also investigated specific genes linked to the development of Dupuytren's, including the gene for TGF-β1. However, studies implicating TGF- β1 with

the medical field [1].

developing the disease.

**3.1. Prevalence**

**3.2. Genetics**

**3. Epidemiology and risk factors**
